中华皮肤科杂志 ›› 2012, Vol. 45 ›› Issue (7): 459-462.

• 论著 •    下一篇

结节性纤维毛囊瘤：一种新的皮肤附属器肿瘤

毛荣军1,韩建德2,房惠琼3,谢乐3,李扬4,李启明3   

  1. 1. 广州中医药大学附属佛山市中医院病理科
    2. 广州市中山大学第一医院皮肤科
    3. 广东佛山 广东中医药大学附属佛山中医院病理科
    4. 广州市中山大学附属第一医院病理科
  • 收稿日期:2012-02-10 修回日期:2012-03-10 出版日期:2012-07-15 发布日期:2012-07-02
  • 通讯作者: 毛荣军 E-mail:mrjys@163.com

Nodular fibrofolliculoma：a new entity of skin adnexal tumor

  • Received:2012-02-10 Revised:2012-03-10 Online:2012-07-15 Published:2012-07-02
  • Contact: Rongjun MAO E-mail:mrjys@163.com

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摘要：

患者女，22岁，左肩部孤立性无痛性肿物逐渐增大2年入院。体检：除左肩部肿块外其余各系统检查未见异常。皮肤科检查：病变局部皮肤隆起，表面光滑，色泽正常，肿物位于皮下，约3.5 cm × 2.0 cm × 1.5 cm，质硬，可移动。局麻下行肿块切除，术中见肿物位于皮下脂肪组织内，结节状，局部具有纤维性假包膜，肿物切面灰白色，质中。镜下，肿瘤组织学形态与乳腺型纤维腺瘤极为相似，呈明显的分叶状，每个小叶由具有管状结构、基底细胞样上皮条索和富于成纤维细胞样细胞的纤维黏液样间质构成，小叶内无毛球和原始毛乳头结构；小叶间为致密的胶原纤维性分隔，其间散在少量炎细胞浸润；多数小叶内基底细胞样上皮条索包绕在纤维黏液样间质周围；部分管状结构内可见少许稀薄并呈同心圆样排列的淡嗜伊红物质。免疫组化显示，小叶内上皮细胞及其管腔内物质强阳性表达细胞角蛋白5/6（CK5/6），不表达CAM5.2及癌胚抗原（CEA），管腔内淡嗜伊红物质糖原染色阴性，支持管状结构为不成熟毛囊，而非汗管。结合临床表现、形态学特点、免疫组化及糖原染色结果，命名为结节性纤维毛囊瘤（nodular fibrofolliculma）。术后随访3月余未见复发。本病是一种毛源性良性附属器肿瘤，具有独特的病理学表现，认为是一种新的皮肤附属器肿瘤。

关键词: 实体肿瘤

Abstract:

A 22-year-old patient was admitted to the hospital with a solitary, gradually growing and painless mass in the left shoulder for 2 years. Physical examination revealed no abnormality except for the skin lesion. Skin examination showed an elevated lesion measuring about 3.5 cm × 2.0 cm × 1.5 cm with smooth surface and normal color, which was located in the subcutaeous tissue, indurated and movable. Resection of the tumor was performed under local anesthesia. On visual observation during operation, the tumor was sited in the subcutaneous fat tissue, nodular-like and surrounded locally by fibrous pseudocapsules with a grey incisal surface and mild texture. Microscopically, the tumor was extremely similar to breast fibroadenoma with multiple lobuli, and each of the lobuli was composed of tubiform structures, basal cell-like epithelial cell trabs and fibromyxoid stroma abundant in fibroblast-like spindle cells. No hair bulb or primitive dermal papillae were observed in the lobuli, which were separated by compact collagen fibers infiltrated by a few scattered inflammatory cells. Fibromyxoid stroma was surrounded by basal cell-like epithelial cell strabs in most lobuli, and some tubiform structures were filled with a little thin lightly eosinophilic material in a concentric arrangement. Immunohistochemistry showed that intralobular epithelial cells were strongly positive for cytokeratin 5/6, but negative for CAM5.2 or carcinoembryonic antigen （CEA）. In addition, the lightly eosinophilic material in lumens was negative for periodic acid-Schiff （PAS） staining. These results suggested that the tubiform structures were immature follicles, but not sweat ducts. The patient was diagnosed with nodular fibrofolliculoma （NFF） based on the clinical manifestations, morphological features, immunohistochemical and PAS staining results. No relapse was observed in more than 3 months of postoperative follow-up. As a benign trichogenic adnexal neoplasm with unique clinicopathological manifestations, NFF may be a new entity of cutaneous adnexal neoplasm.

Key words: entity